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Potential supplementary utility of combined PFA-100 and functional von Willebrand factor testing for the laboratory assessment of desmopressin and factor concentrate therapy in von Willebrand disease.
Blood Coagul Fibrinolysis 2009; 20(6):475-83BC

Abstract

We performed a retrospective audit of cross-laboratory testing of desmopressin and factor concentrate therapy to assess the potential utility of supplementary testing using the PFA-100 with functional von Willebrand factor (VWF) activity testing. Data were evaluated for a large number of patients with von Willebrand disease of type 1, type 2A or type 2M, as well as a comparative subset of individuals with haemophilia or carriers of haemophilia. Laboratory testing comprised pre and postdesmopressin, or pre and postconcentrate, evaluation of factor VIII, VWF antigen (VWF:Ag) and VWF ristocetin cofactor activity as traditionally performed, supplemented with collagen-binding (VWF:CB) testing and PFA-100 closure times. In brief, both therapies tended to normalize VWF test parameters and closure times in individuals with type 1 von Willebrand disease, with the level of correction in closure times related to the level of normalization of VWF, particularly the VWF:CB. However, although occasional correction of closure times was observed in patients with type 2A or type 2M von Willebrand disease, these did not in general normalize PFA-100 closure times either with desmopressin or factor concentrate therapy. In these patients, improvement in closure times was more likely in those in whom VWF:CB values normalized or when VWF:CB/VWF:Ag ratios normalized. This study confirms that there is a strong relationship between the presenting levels of plasma VWF and PFA-100 closure times, and that the supplementary combination of PFA-100 and VWF:CB testing might provide added clinical utility to current broadly applied testing strategies limited primarily to VWF:Ag, VWF ristocetin cofactor and factor VIII:coagulant. Future prospective investigations are warranted to validate these relationships and to investigate their therapeutic implications.

Authors+Show Affiliations

Department of Haematology, Institute of Clinical Pathology and Medical Research (ICPMR), Westmead Hospital, New South Wales, Australia. emmanuel.favaloro@swahs.health.nsw.gov.auNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Evaluation Studies
Journal Article

Language

eng

PubMed ID

19584715

Citation

Favaloro, Emmanuel J., et al. "Potential Supplementary Utility of Combined PFA-100 and Functional Von Willebrand Factor Testing for the Laboratory Assessment of Desmopressin and Factor Concentrate Therapy in Von Willebrand Disease." Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis, vol. 20, no. 6, 2009, pp. 475-83.
Favaloro EJ, Thom J, Patterson D, et al. Potential supplementary utility of combined PFA-100 and functional von Willebrand factor testing for the laboratory assessment of desmopressin and factor concentrate therapy in von Willebrand disease. Blood Coagul Fibrinolysis. 2009;20(6):475-83.
Favaloro, E. J., Thom, J., Patterson, D., Just, S., Baccala, M., Dixon, T., ... Baker, R. (2009). Potential supplementary utility of combined PFA-100 and functional von Willebrand factor testing for the laboratory assessment of desmopressin and factor concentrate therapy in von Willebrand disease. Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis, 20(6), pp. 475-83. doi:10.1097/MBC.0b013e32832da1ad.
Favaloro EJ, et al. Potential Supplementary Utility of Combined PFA-100 and Functional Von Willebrand Factor Testing for the Laboratory Assessment of Desmopressin and Factor Concentrate Therapy in Von Willebrand Disease. Blood Coagul Fibrinolysis. 2009;20(6):475-83. PubMed PMID: 19584715.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Potential supplementary utility of combined PFA-100 and functional von Willebrand factor testing for the laboratory assessment of desmopressin and factor concentrate therapy in von Willebrand disease. AU - Favaloro,Emmanuel J, AU - Thom,Jim, AU - Patterson,David, AU - Just,Sarah, AU - Baccala,Maria, AU - Dixon,Tracy, AU - Meiring,Muriel, AU - Koutts,Jerry, AU - Rowell,John, AU - Baker,Ross, PY - 2009/7/9/entrez PY - 2009/7/9/pubmed PY - 2009/12/23/medline SP - 475 EP - 83 JF - Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis JO - Blood Coagul. Fibrinolysis VL - 20 IS - 6 N2 - We performed a retrospective audit of cross-laboratory testing of desmopressin and factor concentrate therapy to assess the potential utility of supplementary testing using the PFA-100 with functional von Willebrand factor (VWF) activity testing. Data were evaluated for a large number of patients with von Willebrand disease of type 1, type 2A or type 2M, as well as a comparative subset of individuals with haemophilia or carriers of haemophilia. Laboratory testing comprised pre and postdesmopressin, or pre and postconcentrate, evaluation of factor VIII, VWF antigen (VWF:Ag) and VWF ristocetin cofactor activity as traditionally performed, supplemented with collagen-binding (VWF:CB) testing and PFA-100 closure times. In brief, both therapies tended to normalize VWF test parameters and closure times in individuals with type 1 von Willebrand disease, with the level of correction in closure times related to the level of normalization of VWF, particularly the VWF:CB. However, although occasional correction of closure times was observed in patients with type 2A or type 2M von Willebrand disease, these did not in general normalize PFA-100 closure times either with desmopressin or factor concentrate therapy. In these patients, improvement in closure times was more likely in those in whom VWF:CB values normalized or when VWF:CB/VWF:Ag ratios normalized. This study confirms that there is a strong relationship between the presenting levels of plasma VWF and PFA-100 closure times, and that the supplementary combination of PFA-100 and VWF:CB testing might provide added clinical utility to current broadly applied testing strategies limited primarily to VWF:Ag, VWF ristocetin cofactor and factor VIII:coagulant. Future prospective investigations are warranted to validate these relationships and to investigate their therapeutic implications. SN - 1473-5733 UR - https://www.unboundmedicine.com/medline/citation/19584715/Potential_supplementary_utility_of_combined_PFA_100_and_functional_von_Willebrand_factor_testing_for_the_laboratory_assessment_of_desmopressin_and_factor_concentrate_therapy_in_von_Willebrand_disease_ L2 - http://dx.doi.org/10.1097/MBC.0b013e32832da1ad DB - PRIME DP - Unbound Medicine ER -